Postnatal cerebral hemodynamics in infants with severe congenital heart disease: a scoping review

Patients with severe congenital heart disease (CHD) are at risk for impaired neurodevelopment. Cerebral blood supply may be diminished by congenital anomalies of cardiovascular anatomy and myocardial function. The aim of this scoping review was to summarize the current knowledge on cerebral hemodynamics in infants with severe CHD. A scoping review was performed. Five databases were searched for articles published from 01/1990 to 02/2022 containing information on cerebral hemodynamics assessed by neuroimaging methods in patients with severe CHD within their first year of life. A total of 1488 publications were identified, of which 26 were included. Half of the studies used Doppler ultrasound, and half used magnetic resonance imaging techniques. Studies focused on preoperative findings of cerebral hemodynamics, effects of surgical and conservative interventions, as well as on associations between cerebral hemodynamics and brain morphology or neurodevelopment. Cerebral perfusion was most severely affected in patients with single ventricle and other cyanotic disease. Neuroimaging methods provide a large variety of information on cerebral hemodynamics. Nevertheless, small and heterogeneous cohorts complicate this field of research. Further studies are needed to improve our understanding of the link between CHD and altered cerebral hemodynamics to optimize neuroprotection strategies. IMPACT: Postnatal cerebral hemodynamics are altered in infants with congenital heart disease (CHD) as compared to healthy controls, especially in most severe types such as single ventricle or other cyanotic CHD. Associations of these alterations with brain volume and maturation reveal their clinical relevance. Research in this area is limited due to the rarity and heterogeneity of diagnoses. Furthermore, longitudinal studies have rarely been conducted. Further effort is needed to better understand the deviation from physiological cerebral perfusion and its consequences in patients with CHD to optimize neuroprotection strategies

Flow evaluation software for four-dimensional flow MRI: a reliability and validation study

PURPOSE Four-dimensional time-resolved phase-contrast cardiovascular magnetic resonance imaging (4D flow MRI) enables blood flow quantification in multiple vessels, which is crucial for patients with congenital heart disease (CHD). We investigated net flow volumes in the ascending aorta and pulmonary arteries by four different postprocessing software packages for 4D flow MRI in comparison with 2D cine phase-contrast measurements (2D PC). MATERIAL AND METHODS 4D flow and 2D PC datasets of 47 patients with biventricular CHD (median age 16, range 0.6-52 years) were acquired at 1.5 T. Net flow volumes in the ascending aorta, the main, right, and left pulmonary arteries were measured using four different postprocessing software applications and compared to offset-corrected 2D PC data. Reliability of 4D flow postprocessing software was assessed by Bland-Altman analysis and intraclass correlation coefficient (ICC). Linear regression of internal flow controls was calculated. Interobserver reproducibility was evaluated in 25 patients. RESULTS Correlation and agreement of flow volumes were very good for all software compared to 2D PC (ICC ≥ 0.94; bias ≤ 5%). Internal controls were excellent for 2D PC (r ≥ 0.95, p < 0.001) and 4D flow (r ≥ 0.94, p < 0.001) without significant difference of correlation coefficients between methods. Interobserver reliability was good for all vendors (ICC ≥ 0.94, agreement bias < 8%). CONCLUSION Haemodynamic information from 4D flow in the large thoracic arteries assessed by four commercially available postprocessing applications matches routinely performed 2D PC values. Therefore, we consider 4D flow MRI-derived data ready for clinical use in patients with CHD

Signal Thresholding Segmentation of Ventricular Volumes in Young Patients with Various Diseases—Can We Trust the Numbers?

In many cardiac diseases, right and left ventricular volumes in systole and diastole are diagnostically and prognostically relevant. Measurements are made by segmentation of the myocardial borders on cardiac magnetic resonance (CMR) images. Automatic detection of myocardial contours is possible by signal thresholding techniques, but must be validated before use in clinical settings. Biventricular volumes were measured in end-diastole (EDVi) and in end-systole (ESVi) both manually and with the MassK application, with signal thresholds at 30%, 50%, and 70%. Stroke volumes (SV) and cardiac indices (CI) were calculated from volumetric measurements and from flow measured in the ascending aorta and the main pulmonary artery, and both methods were compared. Reproducibility of volumetric measurements was tested in 20 patients. Measurements were acquired in 94 patients aged 15 ± 9 years referred for various conditions. EDVi and ESVi of both ventricles were largest with manual segmentation and inversely proportional to the MassK threshold. Manual and k30 SV and CI corresponded best to flow measurements. Interobserver variability was low for all volumes manually and with MassK. In conclusion, manual and 30% threshold-based biventricular volume segmentation agree best with two-dimensional, phantom-corrected phase contrast flow measurements in a young cardiac referral population and are well reproducible

Myocardial Deformation in Fontan Patients Assessed by Cardiac Magnetic Resonance Feature Tracking: Correlation with Function, Clinical Course, and Biomarkers

Cardiac MR (CMR) is a standard modality for assessing ventricular function of single ventricles. CMR feature-tracking (CMR-FT) is a novel application enabling strain measurement on cine MR images and is used in patients with congenital heart diseases. We sought to assess the feasibility of CMR-FT in Fontan patients and analyze the correlation between CMR-FT strain values and conventional CMR volumetric parameters, clinical findings, and biomarkers. Global circumferential (GCS) and longitudinal (GLS) strain were retrospectively measured by CMR-FT on Steady-State Free Precession cine images. Data regarding post-operative course at Fontan operation, and medication, exercise capacity, invasive hemodynamics, and blood biomarkers at a time interval ± 6 months from CMR were collected. Forty-seven patients underwent CMR 11 ± 6 years after the Fontan operation; age at CMR was 15 ± 7 years. End-diastolic volume (EDV) of the SV was 93 ± 37 ml/m2, end-systolic volume (ESV) was 46 ± 23 ml/m2, and ejection fraction (EF) was 51 ± 11%. Twenty (42%) patients had a single right ventricle (SRV). In single left ventricle (SLV), GCS was higher (p < 0.001), but GLS was lower (p = 0.04) than in SRV. GCS correlated positively with EDV (p = 0.005), ESV (p < 0.001), and EF (p ≤ 0.0001). GLS correlated positively with EF (p = 0.002), but not with ventricular volumes. Impaired GCS correlated with decreased ventricular function (p = 0.03) and atrioventricular valve regurgitation (p = 0.04) at echocardiography, direct atriopulmonary connection (p = 0.02), post-operative complications (p = 0.05), and presence of a rudimentary ventricle (p = 0.01). A reduced GCS was associated with increased NT-pro-BNP (p = 0.05). Myocardial deformation can be measured by CMR-FT in Fontan patients. SLVs have higher GCS, but lower GLS than SRVs. GCS correlates with ventricular volumes and EF, whereas GLS correlates with EF only. Myocardial deformation shows a relationship with several clinical parameters and NT-pro-BNP. Keywords: Biomarkers; CMR; CMR-FT; Fontan; Strai

Right and Left Ventricular Strain Patterns After the Atrial Switch Operation for D-Transposition of the Great Arteries—A Magnetic Resonance Feature Tracking Study

Introduction: Adult survivors of the atrial switch operation for transposition of the great arteries present with a systemic morphologic right ventricle and a subpulmonary morphologic left ventricle. This physiology can be considered a model for the effects of long-term right ventricular pressure overload and of decreased left ventricular afterload. We aimed to determine the impact of these chronically altered loading conditions on myocardial deformation of the ventricles.Materials and methods: Two-dimensional steady state free precession cine images of 29 patients after atrial repair (age 29 ± 7 years) and 19 controls (24 ± 10 years; n.s.) were post-processed with feature tracking software (TomTec 2D CPA). Volumes, ejection fractions, global and free wall longitudinal and circumferential strains of both ventricles were compared between both groups.Results: Systemic right ventricular global longitudinal strain was decreased in patients compared to controls (−12.9 ± 3.3% vs. −18.9 ± 4.6%, p &lt; 0.001), while right ventricular circumferential strain was unchanged (−15.8 ± 3.4% vs. −15.1 ± 5%; n.s.). Left ventricular longitudinal strain was similar in both groups (−17 ± 5.6% vs. −17.5 ± 4.6%; n.s.), but global left ventricular circumferential strain was lower in patients (−20.7 ± 4.1% vs. −27.3 ± 4.5%, p &lt; 0.001). The systemic right ventricle, compared to the systemic left ventricle, showed decreased global longitudinal (p &lt; 0.001) and circumferential strain (p &lt; 0.001). The subpulmonary left ventricle, compared to the subpulmonary right ventricle, demonstrated similar longitudinal (p = 0.223) but higher circumferential strain (p &lt; 0.001).Conclusions: In patients after atrial switch repair for transposition of the great arteries, the systemic right ventricle shows poor longitudinal strain, but maintains normal right ventricular circumferential strain. The left ventricle shows higher circumferential strain than the right ventricle, in both systemic and subpulmonary positions

Magnetic resonance imaging assessment of temporomandibular joint involvement and mandibular growth following corticosteroid injection in juvenile idiopathic arthritis

OBJECTIVE: To assess whether intraarticular corticosteroid injection (CSI) reduces inflammation of the temporomandibular joint (TMJ), prevents growth disturbances of the mandibular condyle, and restores normal growth of the mandibular ramus. METHODS: Retrospective longitudinal magnetic resonance imaging (MRI) evaluation of inflammatory activity, TMJ deformity, and mandibular ramus height in 33 children (23 girls, median age 5.2 yrs) over a median period of 5 years following repetitive CSI to the TMJ. RESULTS: Intraarticular location of CSI led to inflammatory grade improvement in 53% at first MRI followup compared to 20% with extraarticular location (p = 0.005), with more improvement of the mean inflammatory grade after intraarticular CSI (p = 0.001). Rate of osseous deformities of the TMJ deteriorated from 51% at study inclusion to 62% at end of observation period, with progression to severe condylar destruction in 26% of joints including 24% with development of intraarticular calcifications/ossifications. Mean short-term growth rates of the mandibular ramus were negative for intraarticular CSI while positive for extraarticular CSI (p = 0.036). Mean longterm mandibular ramus growth rate (0.7 ± 0.8 mm/yr) after CSI was significantly lower than reported normal mean age- and sex-matched growth rate (1.4 ± 0.1 mm/yr, p < 0.0001). CONCLUSION: Despite improving the inflammatory activity as seen on MRI, repetitive CSI to the TMJ does not reach the treatment goals to prevent progressive osseous deformation and to normalize mandibular ramus growth in children with juvenile idiopathic arthritis

Magnetic Resonance Imaging Follow-up of Temporomandibular Joint Inflammation, Deformation and Mandibular Growth in Juvenile Idiopathic Arthritis Patients on Systemic Treatment

OBJECTIVE To investigate the course of temporomandibular joint (TMJ) inflammation, osseous deformation and mandibular ramus growth in children with juvenile idiopathic arthritis (JIA) during systemic therapy. METHODS Longitudinal study of 38 consecutive JIA patients (29 female, median age 9.0 years, interquartile range 6.2 to 10.7 years) on systemic therapy with TMJ involvement, with two TMJ magnetic resonance imaging (MRI) examinations ≥ 2 years apart and no TMJ corticosteroid injection. Clinical and MRI findings were compared between initial and follow-up examinations and between TMJs with and without active inflammation at baseline. RESULTS Over a median period of 3.6 years (range, 2.0-8.7 years), MRI grade of TMJ inflammation improved (p=0.009) and overall osseous deformity tended to become less severe (p=0.114). In TMJs with arthritis at baseline (46 TMJs), both the grades of inflammation (p<0.001) and deformity (p=0.011) improved. In TMJs with no arthritis at baseline (30 TMJs), the frequency and grade of condylar deformation remained stable. Mandibular ramus growth rates were not significantly different between TMJs with and without arthritis at baseline (1.3 mm/year versus 1.5 mm/year, p=0.273), and were not correlated with the degree of inflammation at baseline or followup. The frequency of facial asymmetry tended to be lower at follow-up than at initial examination (24% versus 45%, p=0.056). CONCLUSION Our results suggest that systemic treatment of TMJ arthritis in children with JIA decreases the degree of inflammation seen on MRI, preserves osseous TMJ morphology and maintains normal mandibular ramus growth

Evolution of posterior fossa and brain morphology after in utero repair of open neural tube defects assessed by MRI

OBJECTIVES: To describe characteristics of foetuses undergoing in utero repair of open neural tube defects (ONTD) and assess postoperative evolution of posterior fossa and brain morphology. METHODS: Analysis of pre- and postoperative foetal as well as neonatal MRI of 27 foetuses who underwent in utero repair of ONTD. Type and level of ONTD, hindbrain configuration, posterior fossa and liquor space dimensions, and detection of associated findings were compared between MRI studies and to age-matched controls. RESULTS: Level of bony spinal defect was defined with exactness of ± one vertebral body. Of surgically confirmed 18 myelomeningoceles (MMC) and 9 myeloschisis (MS), 3 MMC were misdiagnosed as MS due to non-visualisation of a flat membrane on MRI. Hindbrain herniation was more severe in MS than MMC (p < 0.001). After repair, hindbrain herniation resolved in 25/27 cases at 4 weeks and liquor spaces increased. While posterior fossa remained small (p < 0.001), its configuration normalised. Lateral ventricle diameter indexed to cerebral width decreased in 48% and increased in 12% of cases, implying a low rate of progressive obstructive hydrocephalus. Neonatally evident subependymal heterotopias were detected in 33% at preoperative and 50% at postoperative foetal MRI. CONCLUSION: MRI demonstrates change of Chiari malformation type II (CM-II) features. KEY POINTS: Hindbrain herniation is significantly more pronounced in myeloschisis than in myelomeningocele Resolution of hindbrain herniation 4 weeks after in utero closure of ONTD MRI is valuable for preoperative assessment and postoperative evaluation following in utero repair

Normal myocardial native T1_{1} values in children using single-point saturation recovery and modified look-locker inversion recovery (MOLLI)

BACKGROUND T$_{1}$ mapping is useful to quantify diffuse myocardial processes such as fibrosis, edema, storage disorders, or hemochromatosis. Normal pediatric myocardial T$_{1}$ values are scarce using modified Look-Locker inversion recovery (MOLLI) sequences and unavailable using Smart1Map, a single-point saturation recovery sequence that measures true T$_{1}$ . PURPOSE/HYPOTHESIS To establish normal pediatric myocardial T$_{1}$ values by Smart1Map and to compare them with T$_{1}$ by MOLLI. STUDY TYPE Prospective cohort study. SUBJECTS Thirty-four children and adolescents aged 8-18 years (14 males) without cardiovascular or inflammatory diseases. FIELD STRENGTH/SEQUENCES 1.5T, MOLLI, Smart1Map. ASSESSMENT Mean T$_{1}$ values of the left ventricular myocardium, the interventricular septum, and the blood pool were measured with MOLLI and Smart1Map in basal, mid-ventricular, and apical short axis slices. STATISTICAL TESTS T$_{1}$ values were compared between locations and methods by paired samples t-tests, Wilcoxon signed ranks test, repeated-measures analysis of variance (ANOVA), or Friedman's test. Pearson's correlation coefficient was calculated. For interobserver variability, intraclass correlation coefficients and coefficients of variation were calculated, and Bland-Altman analyses were performed. RESULTS T$_{1}$ values were longer by Smart1Map than by MOLLI in all measured locations (myocardium: 1191-1221 vs. 990-1042 msec; all P 0.05) by either method. Septal vs. total myocardial T$_{1}$ values in each slice did not differ by MOLLI (basal P = 0.371; mid-ventricular P = 0.08; apical P = 0.378) nor by Smart1Map (basal P = 0.056; mid-ventricular P = 0.918; apical P = 0. 392), after artifacts had been carefully excluded. DATA CONCLUSION We established pediatric normal native T$_{1}$ values using the Smart1Map sequence and compared the results with T$_{1}$ mapping with MOLLI. Septal T$_{1}$ values did not differ from total myocardial T$_{1}$ values in each of the myocardial slices. LEVEL OF EVIDENCE 2 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2019